IMPORTANCEAdult patients with congenital heart disease (CHD) have an increased incidence of cancer, presumably owing to repeated radiation exposure, genetic predisposition, or repeated stress factors during heart interventions. However, there are limited data on the risk of cancer in children and young adults with CHD compared with the general population. OBJECTIVE To determine the risk of developing cancer from birth to age 41 years among patients with CHD compared with healthy matched controls. DESIGN, SETTING, AND PARTICIPANTS This registry-based, matched, prospective cohort study in Sweden used data from the Patient and Cause of Death Registers. Successive cohorts of patients